Neurotrophic keratitis can be very challenging to manage in the pediatric population. Tarsorrhaphy as a treatment option may contribute to amblyopia and may be unacceptable cosmetically. Clinicians may be reluctant to resort to therapeutic contact lens in cases of reduced corneal sensation in children due to risk of infection. Our purpose is to report the outcome of five pediatric cases of neurotrophic keratitis managed with prosthetic replacement of the ocular surface ecosystem (PROSE) treatment. PROSE treatment utilizes custom-designed and fabricated prosthetic devices that are applied and removed on a daily basis to support ocular surface functions in complex corneal disease.

Methods

Retrospective review of medical records of five patients age 13 years or less who were referred to a single center for PROSE treatment after failure of conventional measures for treatment of congenital or acquired neurotrophic keratitis.

Results

Ages ranged from 3 to 13 years at initial consultation for PROSE treatment. Three patients were female and two were male. Neurotrophic keratitis was unilateral in 4 cases and bilateral in 1 case. Underlying basis for neutrotrophic keratitis was familial dysautonomia (1 case), resection of Arnold-Chiari malformation (2 cases) resection of hemangioma (1 case), and idiopathic/presumed herpes simplex virus (1 case). Three eyes in three children entered with epithelial breakdown, and were managed with daily wear of PROSE device and ointment at night. Best corrected vision at entry for older children ranged from 20/200 to 20/40 and improved to range of 20/50 to 20/20. For the two children age 3 at entry, vision was fix and follow each eye (1 patient) and 20/130 improving to 20/94 after 2 months (1 patient). In every eye there was reduction of staining and epithelial irregularity. In one case there was reduction of corneal opacity with concomitant topical steroid use. There were no complications of microbial keratitis during documented wear of 6 months to 10 years, including one patient in which there was 1 year of concomitant topical steroid use.

Conclusions

PROSE treatment is a useful option for preserving corneal integrity and vision in pediatric cases of neurotrophic keratitis.